Download Vasculitis Review: Intern Conference

Vasculitis Review:
Intern Conference
Jason Haag
Overview
• Nomenclature of vasculitis
• Overview of most important diseases
• Quiz review
Nomenclature
• Vasculitis: Inflammation on blood vessels
causing vessel and tissue damage
• Classification is according to the Chapel Hill
Consensus Conference of 1994
• Large-vessel vasculitides (2)
• Medium-vessel vasculitides (3)
• Small-vessel vasculitides (many)
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ANCA-associated (5)
Non-ANCA-associated (many)
Jennette and Falk, Curr Op Rheumatol, 2007
Large Vessel Vasculitides (2)
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Giant cell arteritis (formerly called
temporal arteritis)
Takayasu’s arteritis
Distinguished only by age of pt > or < 50 yo
Both involve the aorta and its primary
branches
Medium Vessel Vasculitides (3)
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Polyarteritis nodosa (PAN)
Buerger’s = Thromboangiitis obliterans
Kawasaki’s disease = Mucocutaneous LN
syndrome
Small Vessel Vasculitides (many)
• ANCA-associated (5)
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Wegener’s granulomatosis (WG)
Churg-Strauss syndrome (CSS)
Microscopic polyangiitis (MPA) – no granulomas
Drug-induced ANCA vasculitis
Idiopathic
Small Vessel Vasculitides (many)
• Non-ANCA-associated (many)
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Infectious (Hep B, Hep C, HIV, Parvo, CMV, EBV)
CVD-related (RA, SLE, Bechet’s, Sjogren’s)
Drug-related (cutaneous leukocytoclastic vasculitis)
Henoch-Schonlein Purpura (HSP) – IgA deposits
Goodpasture’s disease
Cryoglobulinemia
IBD-related
Urticarial vasculitis
Paraneoplastic
Large Vessel Vasculitis:
Takayasu’s arteritis
• Affects women < 40 yo
• Prepulseless phase: systemic sx
• Pulseless phase: arm claudication, HA,
dizziness, visual changes, decreased arm
pulses or pressure differential,
subclavian/abdominal bruits
• Tx with Prednisone
Takayasu’s arteritis
MRA
Large Vessel Vasculitis:
Giant Cell Arteritis
• Sx: fatigue, HA, visual changes, scalp
tenderness, jaw claudication
• Often associated with PMR (pain and
stiffness in neck, shoulder, pelvic girdles)
• Prompt corticosteroids prevent blindness
Medium Vessel Vasculitis:
Polyarteritis Nodosa (PAN)
• Peaks in 5th decade, slight male
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preponderance
Sx: constitutional sx, skin lesions (purpura,
livedo reticularis, ulcers, nodules),
mononeuritis multiplex, hematuria, abdominal
pain, testicular pain
Aneurysms and narrowing on MRA
Associated with Hep B
Tx with Prednisone, cyclophosphamide
Polyarteritis Nodosa (PAN)
Medium Vessel Vasculitis:
Buerger’s Disease
 Young, 20-40 y.o
 Male
 Inflammation/thrombosis in
hands/feet  pain
 Recent or current tobacco use
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Tx with smoking cessation
Medium Vessel Vasculitis:
Kawasaki’s Disease
 Classic Dx
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5 days of fever + 4 of 5 criteria
 Erythema/cracking
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of lips
strawberry tongue
 Trunkal
rash
 Swelling of hands/feet
 Conjuctivitis
 Cervical LAD
 Also can have joint pain, coronary
aneurysms, myocarditis
 Tx: IVIG
Medium Vessel Vasculitis:
Kawasaki’s Disease
Small Vessel Vasculitis:
Churg-Strauss Syndrome
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Middle-aged adults
Asthma, eosinophilia (>10%), neuropathy (mono- or
poly- in stocking/glove), pulmonary infiltrates
ANCA+ vasculitis
Tx: Prednisone, cyclophosphamide
Small Vessel Vasculitis:
Wegener’s Granulomatosis
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Granulomas in upper resp tract (sinusitis, otitis,
epistaxis), lower resp tract (hemoptysis), and kidneys
(GN), can involve eye and nose
Tx: Stx, cyclophosphamide
Small Vessel Vasculitis:
Goodpasture’s Syndrome
 Hemoptysis/hematuria
 Linear IgG deposits
across basement
membrane
 Tx:
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Stx, plasmapheresis
Small Vessel Vasculitis:
Bechet’s Syndrome
• Highest in Iran, Turkey, and Japan
• Recurrent oral and genital ulcers
• Uveitis causes blindness in 10%
• Skin pathergy
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> 2mm nodule after 48 hours
• Multiple systems involve
• Venous thrombosis
• Tx: Stx, TNF blocker
T = 24h
T= 48h
T= 0
Small Vessel Vasculitis
Henoch Schonlein Purpura
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Most common in children aged 3-15, but also occurs in
adults
Presents with classic tetrad of purpuric rash,
abdominal pain, renal disease, and arthralgias
Can progress to renal failure (adults)
Tx: Pain control, Stx if neccessary
Small Vessel Vasculitis:
Cryoglobulenemia
 Nonspecific Sx
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Arthralgias, myalgias, fever,
glomerularneprhitis
Palpable pupura
 Associated with
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Hep C, RA, SLE
 Low complement levels
 Tx primary disease
Quiz Review